Form Of Chronic Inflammatory Demyelinating Polyneuropathy Research Articles

Evolving Immunologic Perspectives in Chronic Inflammatory Demyelinating Polyneuropathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the commonest chronic idiopathic dysimmune neuropathy. Pathophysiologic processes involve both cellular and humoral immunity. There are various known forms of CIDP, likely caused by varying mechanisms. CIDP in its different forms is a treatable disorder in the majority of patients. The diagnosis of CIDP is clinical, supported routinely by electrophysiology. Cerebrospinal fluid analysis may be helpful. Routine immunology currently rarely adds to the diagnostic process but may contribute to the identification of an associated monoclonal gammopathy with or without hematologic malignancy and the consideration of alternative diagnoses, such as POEMS syndrome, anti-myelin associated glycoprotein (MAG) neuropathy or chronic ataxic neuropathy, with ophthalmoplegia, M-protein, cold aglutinins and disialosyl antibodies (CANOMAD). The search for antibodies specific to CIDP has been unsuccessful for many years. Recently, antibodies to paranodal proteins have been identified in a minority of patients with severe CIDP phenotypes, often unresponsive to first-line therapies. In conjunction with reports of high rates of antibody responses to neural structures in CIDP, this entertains the hope that more discoveries are to come. Although still arguably for only a small minority of patients, in view of current knowledge, such progress will enable earlier accurate diagnosis with direct management implications but only if the important, unfortunately and infrequently discussed issues of immunologic technique, test reliability and reproducibility are adequately tackled.

P72-S Recurrent proximal motor conduction blocks in a patient with relapsing-remitting predominantly motor form of CIDP

Background Chronic inflammatory demyelinating polyneuropathy (CIDP) is a clinically heterogeneous autoimmune disease. Motor-dominant form of CIDP is frequently presented as relapsing-remitting course. Although motor conduction blocks have to be the pathophysiological substrate in these cases, there is no many descriptions in literature. Material and Methods We report a male patient (63 years old) with relapsing-remitting-CIDP, with 4 episodes of acute motor weakness that responded well to endovenous inmunoglobulin treatment. Results Electrophysiological studies showed diffuse demyelinating changes in motor conduction studies in upper and lower limbs, and minor sensory conduction abnormalities. Relapsing-remitting motor conduction blocks were demonstrated in proximal segments of upper limb nerves, in parallel with improvement and worsening clinical changes. Conclusions Recurrent proximal motor conduction blocks are demonstrated in a patient with relapsing-remitting predominantly motor form of CIDP. Differential diagnosis and the pathophysiological basis is discussed.

Clinical heterogeneity in mild chronic inflammatory demyelinating polyneuropathy

We describe the clinical presentation, progression and electrodiagnostic features of three patients with a mild form of chronic inflammatory demyelinating polyneuropathy (CIDP). The unusually mild but also variable clinical picture was a cause of diagnostic uncertainty in all, but CIDP was eventually confirmed by extensive electrophysiological studies in each case, as well as by histology in one. Cerebrospinal fluid protein was raised in only one patient. Two patients were treated by intravenous immunoglobulins and both improved. Awareness of the existence of this relatively benign form of CIDP in its various presentations is essential as it can be functionally disabling, progress to more severe symptomatology, and as patients may benefit from immunomodulatory therapy.